Prosthetic dental management of a patient with anhidrotic ectodermal dysplasia: a case report / Manejo protésico dental de un paciente con displasia ectodérmica anhidrótica: reporte de caso

Introduction: Ectodermal dysplasia (ED) comprises a broad group of genetic disorders characterized by alterations of the structures derived from the ectoderm, including those of the stomatognathic system. Case Report: The present article aims to report the prosthetic management of a patient with anhidrotic ectodermal dysplasia. A male patient diagnosed with ED who attended the dental consultation displaying oligodontia; underdeveloped alveolar ridges were observed. Results: The established treatment consisted of the adaptation of implant-supported fixed full-arch prosthesis designed through CAD-CAM technology for the lower jaw and of a removable partial prosthesis with muco-dental support for the upper jaw. The dental approach of patients with ED is based on a correct analysis of the facial characteristics and stomatological conditions of each subject. Conclusion: A multidisciplinary approach is mandatory due to the biological and functional complexity in biomechanical terms of these individuals.

Introducción: La displasia ectodérmica (DE) comprende un amplio grupo de trastornos genéticos caracterizados por alteraciones de las estructuras derivadas del ectodermo, incluidas las del sistema estomatognático. Reporte de Caso: El presente artículo tiene como objetivo informar del manejo protésico de un paciente con displasia ectodérmica anhidrótica. Paciente varón diagnosticado de DE acudió a consulta odontológica por oligodoncia; Se observaron crestas alveolares subdesarrolladas. Resultados: El tratamiento establecido consistió en la adaptación de una prótesis de arcada completa fija implantosoportada diseñada mediante tecnología CAD-CAM para el maxilar inferior y de una prótesis parcial removible con soporte muco-dental para el maxilar superior. El abordaje odontológico de los pacientes con DE se basa en un correcto análisis de las características faciales y condiciones estomatológicas de cada sujeto. Conclusión: Un enfoque multidisciplinario es obligatorio debido a la complejidad biológica y funcional en términos biomecánicos de estos individuos.

Esthetic rehabilitation in primary and permanent successors incisors in a child with conoidal and absence teeth: a case report

Objective: To show the aesthetic treatment performed in deciduous incisors and in permanent successors in a child with congenital dental abnormalities (conoid and missing teeth), which associated with bone disproportion of the middle third of the face led to a suspicious diagnosis of ectodermal dysplasia. Case report: This report was written following the CARE Statement. A 6-year-old girl attended the Pediatric Dental Clinic complaining about bullying due to appearance her teeth. The reanatomization of the conoid-shaped deciduous incisors was done with direct composite resin using preformed acetate crowns. After 15 months, the deciduous incisors had exfoliated and the permanent incisors erupted also with a conoid shape. For aesthetic rehabilitation, direct composite restorations were performed using the incremental technique guided by a silicone matrix made based on the diagnostic wax-up. Conclusion: In the follow-up, the patient and guardians reported satisfaction with appearance of her smile and an increased self-esteem. The case remains under follow-up and future planning will include orthodontics and prosthodontics.

Objetivo: Mostrar o tratamento estético realizado em incisivos decíduos e em seus sucessores permanentes em uma criança com anomalias dentárias congênitas (dentes conoides e ausentes), que associado a desproporção óssea do terço médio da face levaram a um diagnóstico suspeito de displasia ectodérmica. Relato de caso: Este relato foi redigido seguindo o CARE Statement. Menina de 6 anos de idade compareceu à Clínica de Odontopediatria com queixa de bullying devido à aparência de seus dentes. A reanatomização dos incisivos decíduos conoides foi feita com coroas de acetato pré-formadas e resina composta direta. Após 15 meses, os incisivos decíduos esfoliaram e os incisivos permanentes irromperam também com formato conoide. Para a reabilitação estética, foram realizadas restaurações diretas com resina composta pela técnica incremental guiada por matriz de silicone confeccionada a partir do enceramento diagnóstico. Conclusão: No seguimento, paciente e responsáveis relataram satisfação com a aparência do sorriso e aumento da autoestima. O caso continua em acompanhamento e o planejamento futuro incluirá ortodontia e prótese dentária.

Rehabilitación inicial de un niño con anodoncia: presentación de una situación clínica / Initial rehabilitation of a child with anodontia: report of a clinical situation

El objetivo de este trabajo es describir la rehabilitación inicial de un niño preescolar con displasia ectodérmica (DE) y anodoncia. Realizó la primera consulta a los 30 meses con ambos rebordes desdentados y requerimientos familiares de estética y función acorde a su edad. Requirió un amplio abordaje interdisciplinario (odontopediatra, psicóloga, fonoaudióloga y especialista en prótesis). Se instalaron prótesis totales removibles (PTR) en ambos maxilares rebasadas con material resiliente de larga duración, que cumplieron los requisitos de retención, soporte, estabilidad y confort para el niño.Conclusión: los pacientes con anodoncia requieren rehabilitación a edades tempranas, siendo necesaria la participación de equipos interdisciplinarios que aportan diferentes recursos para mejorar la estética, favorecer la función y la inserción en el contexto psicosocial acompañando el crecimiento y desarrollo (AU)

Dental Management and Prosthetic Rehabilitation of Patients Suffering from Hypohidrotic Ectodermal Dysplasia: A Report of Two Case Histories.

The prosthetic restoration and dental long-term care of two oligodont male cousins suffering from X-linked hypohidrotic ectodermal dysplasia is described in two case histories. The first patient was three times supplied with removable dentures at an age from 5 to 12 years, and the second patient was restored twice by tooth- and implant-supported dentures within an observation period of 10 years. In both patients, implants were placed in the growing jaw (both arches) in order to enhance denture retention by single attachments and/or a palatinal bar. In one patient, loss of one maxillary implant occurred 3 years after implant exposure and 2 years after the provision of the implant-supported denture. In the second patient, after completion of growth and preceding bone augmentation, further implants facilitated an improved removable maxillary restoration. In both patients, an orthodontic alignment and reshaping of the conical anterior teeth by means of resin or full-ceramic crowns was applied to improve the esthetic appearance. Patients with HED require a consistent lifelong attendance comprised of oral hygiene, denture maintenance, and refitting. In oligo-/anodont children, the placement of implants in selected regions may be useful for the enhancement of denture retention. However, due to poor bone quality and volume, implant failure may occur.

Rehabilitation of ectodermal dysplasia patients presenting with hypodontia: outcomes of implant rehabilitation part 1.

PURPOSE: This study has evaluated the pre and post perceptions of patients with ectodermal dysplasia (ED) who have been referred to Westmead Centre for Oral Health for treatment with dental implants. METHODS: Six patients with ED and hypodontia were treated with new maxillary complete dental prostheses or fixed implant prostheses, and a mandibular fixed dental prosthesis with implants. Patient perceptions were recorded with a 10cm visual analogue scale (VAS). All patients were asked to draw a line on the VAS which best described their feelings regarding aesthetics, chewing, diet and speech. RESULTS: Improvement in aesthetics was reported for all patients with change scores ranging from +9 to +4 points. Similarly, all patients reported an improvement in chewing ability with +6 point changes in 2 patients, and +8, +3, +7 and +1 in the other 4 patients. Five out of 6 patients reported less dietary restriction with 3 patients each indicating a change of +6 points, +4 points, +3 points, and 2 patients reporting +1 point change. Speech improved by +1 point for 3 patients, whilst 1 patient improved by +6 points, a further 2 patients scored no change. CONCLUSIONS: Patients with ED treated with dental implants reported encouraging outcomes post treatment after prostheses were fitted. Follow-up from 1.6 to 6.8 years has confirmed these improvements.

Immediate Vacuum Formed Overdenture for a Pediatric Patient with Ectodermal Dysplasia.

Restoring the dental and facial esthetics in a growing patient with ectodermal dysplasia (ED) is fundamental to improve psychosocial issues, esthetics, and function. The purpose of this clinical report was to present a simple, fast, and cost-effective technique to re-establish a pleasant smile of an eight-year-old male patient with hypohydrotic ED. A vacuum-formed overdenture prosthesis is described, which is recommended as an immediate interim restorative treatment in the maxillary arch with excellent stability and retention.

Prosthodontic rehabilitation with monolithic, multichromatic, CAD-CAM complete overdentures in an adolescent patient with ectodermal dysplasia: A clinical report.

Ectodermal dysplasia is a rare, hereditary, congenital disease that affects the normal development of certain tissues and structures of ectodermal origin. The disease is manifested to different degrees of severity and may involve the nose, eyes, hair, nails, sweat glands, and enamel. This report describes a 14-year-old boy with ectodermal dysplasia, rehabilitated with monolithic, multichromatic maxillary and mandibular computer-aided design and computer-aided manufacturing (CAD-CAM) acrylic resin complete overdentures.

Prosthodontic Rehabilitation of an Ectodermal Dysplasia Patient with Implant Telescopic Crown Attachments.

Telescopic crown attachments have been successfully used in completely and partially edentulous patients. This type of attachment provides retention, support, and stability with optimal hygiene for the removable dental prosthesis (RDP). With the emergence of CAD/CAM technology, telescopic crown attachments can be virtually designed and milled precisely to ensure a passive fit of the attachment parts and maximal functionality of the RDP. This article reviews both the clinical outcome and prosthodontic rehabilitation of telescopic crown attachments of an edentulous ectodermal dysplasia patient with solitary rigid conical telescopic crown attachments.

[Masticatory performance and assessment of life quality of children with ectodermal dysplasia after prosthetic rehabilitation].

OBJECTIVE: To evaluate masticatory performance and life quality of children with ectodermal dysplasia (ED) after prosthetic rehabilitation. METHODS: Six children with ED received denture restoration and 18 healthy children were involved in this study. The surface electromyography (EMG) of masseter (MM) and anterior temporalis (TA) during clenching and chewing movement were recorded. The EMG amplitude, area, asymmetry index of total and activity index of MM/TA were compared at each stage. The masticatory efficiency was measured with spectrophotometer. The life quality was assessed using visual analogue scale questionnaire. RESULTS: The EMG amplitude of MM and TA during chewing in ED Group were 41.7% and 45.6% of the control group respectively, the area were 35.9% and 36.0% respectively. Significant difference in asymmetry index of total during clenching was observed between the two groups (P < 0.05) but not during chewing (P > 0.05). The differences of activity index of MM/TA during clenching and chewing between the two groups were not detected (P > 0.05). The masticatory efficiency of ED group was 67.2% of the control group. The score of chewing function in children with ED after prosthetic rehabilitation was three times higher than before, and no difference was present between the two groups (P > 0.05). CONCLUSIONS: Early prosthetic rehabilitation can significantly improve the masticatory performance and life quality of children with ED.

Influence of oral rehabilitation on the oral health-related quality of life of a child with ectodermal dysplasia.

Ectodermal dysplasia (ED) is a rare congenital hereditary disorder among a group of syndromes characterized by abnormalities of ectodermic structures. The purpose of this report is to compare the oral health-related quality of life (OHRQoL) before and after complete oral rehabilitation of a five-year-old boy with ED. Delivery of upper and lower dentures resulted in immediate improvement of the child's OHRQoL. Although ED affects patients physically and emotionally, the early oral rehabilitation of young patients is crucial to improve their social interaction and restore their speech and masticatory function.

A multidisciplinary treatment of patients with craniofacial disorders. Own experience.

Oral rehabilitation of patients with craniofacial disorders is a great challenge and needs a multidisciplinary approach. This is due to the diverse etiology of the disease and severity of changes in tissues and organs. Congenital absence of tooth germs manifested in the form of oligodontia or anodontia, the presence of persistent deciduous teeth in ectodermal dysplasia (ED), cleft lip and hard palate or cancer-induced changes in the tissues of the stomatognathic system are the most common causes of these disorders. The observed abnormalities are responsible for functional disorders of musculo-articular system, speech and chewing. In addition, noticeable adverse changes in the appearance have a huge psychological impact on patients and their well-being. Therefore, the treatment of these medical conditions should begin in childhood and comprise interdisciplinary rehabilitation, involving prosthetics and orthodontics supported by surgery, as well as speech or laryngological therapy. In this paper the interdisciplinary treatment of patients with oral hard and soft tissues disorders during ectodermal dysplasia is discussed. Early oral rehabilitation can restore lost or abnormally shaped tissues and proper functions of the masticatory system. It can also have a positive impact on further physical and psychological development of patients.

Using implants for prosthodontic rehabilitation of a 4-year-old with ectodermal dysplasia.

Ectodermal dysplasia (ED) is an inherited disorder that affects ectodermally derived organs, such as teeth. Pathogenesis is thought to involve an altered epithelium-mesenchymal interaction. ED patients have oligodontia (or sometimes anodontia) in addition to other abnormalities involving the skin, sweat glands, or hair. Many different subtypes have been introduced in the literature. This article describes the case of a 4-year-old patient who, after being diagnosed with ED, was put on a treatment plan that involved mandibular implants, reshaping of the maxillary primary central incisors, and prosthetic dental rehabilitation. Due to the child's rapid growth, both dentures were changed 9 months post-treatment. Two years post-treatment, the maxillary denture was changed again and the child was placed under close supervision.

Full mouth rehabilitation of a young patient with partial expressions of ectodermal dysplasia: a clinical report.

Ectodermal dysplasia (ED) is a hereditary disorder characterized by the abnormal development of specific tissues and structures of ectodermal origin. This clinical report describes the multidisciplinary approach to the diagnosis and treatment of a 24-year-old patient with partial expression of ED. The treatment plan used dental implants to support fixed prostheses and ceramic restorations to establish acceptable esthetics and provide function.

From childhood to adulthood: oral rehabilitation of a patient with ectodermal dysplasia.

Ectodermal dysplasia, in all its varieties, occurs in approximately 1 in every 100,000 live births. Depending on the variety, hypohidrosis, hypotrichosis, hypodontia, oligodontia, and/or anodontia may be exhibited. Few long-term clinical reports exist. This report follows the development, growth, and initial treatment of a 10-year-old patient with ectodermal dysplasia and his subsequent oral rehabilitation 20 years later.

Rehabilitation of children with ectodermal dysplasia. Part 1: an international Delphi study.

PURPOSE: An international Delphi study was undertaken to determine by consensus an agreed approach to the management of children with dental manifestations of ectodermal dysplasia, including the use of dental implants. This was done using a questionnaire developed by an interdisciplinary team. MATERIALS AND METHODS: The Delphi study questionnaire was built around 19 areas of clinical relevance and included 90 items. Topic areas included dental disability; initial diagnosis; global disability; oral health aspects of dental treatment (orthodontics, hypodontia, anodontia, implants); and case studies of selected treatment options. Eleven teams from six countries contributed to three iterations of the questionnaire. An algorithm was designed to standardize analysis of the questionnaire answers, all of which were blinded to ensure anonymity. The second and third rounds of the questionnaire excluded previously agreed-upon items but included the responses to the questions from the earlier rounds. The nonconsensus items inquired about the use of radiographs at initial diagnosis; sedation of an uncooperative child; use of a pretreatment questionnaire; the age range for specific treatments (eg, dentures, orthodontics, implants); specific uses of implants (eg, partial prostheses, overdentures, cantilevered prostheses); and case study 2. The residual nonconsensus questions were subsequently discussed at a 2-day meeting. RESULTS: Among the 90 questions and partial questions, there was progressive consensus, with agreements in rounds 1, 2, and 3 of 61%, 21%, and 8%, respectively. At the conclusion of round 3, there was 90% agreement and it was considered that the nonconsensus items required in-depth face-to-face discussion at a consensus meeting, which is described in part 2 of the study. CONCLUSION: The Delphi study provided an opportunity to engage specialist teams in recognized centers to integrate their clinical knowledge and draw on published data to develop a consensus of evidence-based responses.

Rehabilitation of children with ectodermal dysplasia. Part 2: an international consensus meeting.

A consensus meeting was arranged to provide an opportunity to discuss the residual nonconsensus questions following three rounds of a Delphi study. It was hoped that the nonagreements could be resolved to define a comprehensive protocol for the management of ectodermal dysplasia, particularly with respect to the use of dental implants in growing patients. An international panel of expert clinicians in pediatric dentistry, prosthodontics, and orthodontics was invited to be part of the Delphi study to develop agreement on clinical questions through a consensus of ideas. Each expert had been invited to form a study group or team within his or her home institution. As required by the Delphi protocol, a 90-part questionnaire was considered by the collaborating teams and progressed through three iterations with increasing agreement. This process is discussed in part 1 of the study. The residual nonconsensus questions, which represented 10% of the questionnaire, required collaborative interaction for resolution. The consensus meeting was held in London, England, over a 2-day period with support from Nobel Biocare and the British Dental Association.

[Hypodontia due to ectodermal dysplasia: rehabilitation with very early dental implants]. / Hypodontie d'une dysplasie ectodermique: traitement par implantologie très précoce.

INTRODUCTION: Anhidrotic ectodermal dysplasia is a rare disease combining hypodontia, hypotrichosis, and hypohidrosis. The quality of life is greatly impaired very early, with major difficulty for feeding and drawbacks of usual dental prostheses. Early implant placement is a therapeutic alternative. It is usually performed in areas of stable growth, such as the mandibular symphysis. We report the case of very early implant placement in a child presenting with hypodontia related to ectodermal dysplasia. CASE PRESENTATION: A 6-year-old male patient was treated with maxillary and mandibular implant-borne prosthetic rehabilitation. Five implants were placed in the mandible and seven in the maxilla. The esthetic and functional outcome was satisfactory, improving the quality of life. DISCUSSION: Very early implant-borne prosthetic rehabilitation is an alternative, which could become a first line treatment. It restores oro-facial functions allowing for a better development of maxillo-facial bones. This alternative is not without risks. But is it acceptable to wait until teenage with an inadequate removable prosthesis, because growth is not completed?

Avaliação da qualidade de vida de portador de displasia ectodérmica reabilitado com implantes osseointegrados: acompanhamento clínico e radiográfico de 5 anos / Assessment of life quality of a patient with ectodermal dysplasia rehabilitated with osseointegrated implants: five years clinical and radiographic follow-up

A displasia ectodérmica é uma desordem genética associada a anormalidades dentárias que resultam em severos problemas funcionais, estéticos e de qualidade de vida. Pacientes com displasia ectodérmica comumente sofrem com a ausência total ou parcial dos dentes, que ocasiona uma alteração nos padrões normais de desenvolvimento dos maxilares. Para reabilitação desses pacientes, os implantes dentários têm sido cada vez mais utilizados devido à severidade da doença limitar outras opções de tratamento reabilitador. Esse artigo traz um relato de caso com avaliação pré e pós-operatória da qualidade de vida de uma paciente com 55 anos de idade afetada por displasia ectodérmica que exibiu oligodontia e atrofia severa dos maxilares. Ela foi tratada com implantes osseointegrados, seguindo a filosofia de função imediata, baseada na filosofia do sistema Novum® em mandíbula e com a técnica All-on-4® híbrida em maxila, sendo 3 implantes zigomáticos e 1 convencional, apresentando follow-up de 5 anos.

Ectodermal dysplasia is a genetic disorder associated with dental anomalies that severely affect function, aesthetics and quality of life. Patients with ectodermal dysplasia often have complete or partial anodontia, which affects normal jaw development. Dental implants have increasingly been used for the rehabilitation of these patients because their disease severity reduces treatment options. This report describes the pre- andpostoperative quality of life of a 55-year old woman with ectodermal dysplasia that presented with hypodontia and severe atrophy of the jaws. She was treated with dental implants for immediate functional loadingusing the Novum® protocol for the mandible and the All-on-4® hybrid technique in the maxilla, together with zygomatic implants and 3 conventional implants. The patient was followed up for 5 years.

Rehabilitación protésica en displasia ectodérmica hipohidrótica: reporte de un caso / Prosthetic rehabilitation in hypohidrotic ectodermal dysplasia: Report of a case

La displasia ectodérmica hipohidrótica es un síndrome genético, caracterizado principalmente por hipohidrosis, hipotricosis e hipodoncia. Esta enfermedad es asociada con hipodoncia y atrofia de los procesos alveolares. El aspecto facial y la ausencia dental múltiple puede ser causa de problemas de socialización por parte de los niños afectados. El propósito de este reporte es describir las características y el manejo odontológico y protésico de un paciente masculino de 5 años de edad diagnosticado con el síndrome.

Hypodriotic ectotermal dysplasia is a genetic syndrome characterized mainly by hypodriosis, hypotrichosis and hypodontia. This disesase is associated with hypodontia and atrophy of the alveolar process. The facial aspect and the multiple missing teeth can cause problems of socialization of the affected children. The aim of this report is to describe features and dental and prosthetic management of a 5-year-old boy, diagnosed with the syndrome.

Implant treatment in grafted and native bone in patients affected by ectodermal dysplasia.

Ectodermal dysplasia (ED) is a congenital syndrome characterized chiefly by abnormalities of tissues of ectodermal origin, namely skin, nails, hair, and teeth. Dental treatment of patients with ED is necessary because it affords the opportunity to develop normal forms of speech, chewing, swallowing, and normal facial support. Because there are few reports focusing on implants inserted in bone grafted in patients with ED, we therefore performed a retrospective study on 44 implants inserted in 4 patients to detect those variables acting on survival and crestal bone remodeling around implant neck in such subjects. Forty-four fixtures were analyzed. Several patient-related (age and sex), anatomic (maxilla and mandible and tooth site), implant (type, length, and diameter), surgical (sites and types of grafts), and prosthetic (type of loading) variables were investigated. Implant failure and peri-implant bone resorption were considered as predictors of clinical outcome. Kaplan-Meier algorithm and Cox regression were then performed to detect those variables statistically associated with the clinical outcome. Implant length and diameter ranged from 11.5 to 15 mm and from 3.5 to 4.0 mm, respectively. Implants were inserted to replace 12 incisors, 12 cuspids, 11 premolars, and 9 molars. No implant was lost. On the contrary, implant's length, grafted sites, and type of loading affected univariate analysis, but these data were not confirmed by multivariate algorithm. Dental implants and bone grafts to orally rehabilitate patients with ED are valuable devices with no difference if compared with unaffected patients, at least in adults.